Introduction: Vein of Galen aneurysmal malformation (VGAM) is a rare, congenital cerebrovascular malformation with high morbidity and mortality. Parameters to foresee clinical progression and allow individualized parent counseling are lacking. The aim of this study was to evaluate aortic steal measured by Doppler ultrasound as a prognostic parameter in these neonates. Methods: A retrospective monocentric analysis of cardiac ultrasound exams before embolization in neonates with VGAM was conducted. Percentage of aortic steal measured by time-averaged maximum velocity above and below the zero flow baseline by pulsed Doppler ultrasound at the preductal aortic isthmus was calculated. Association of aortic steal with parameters of acute organ dysfunction (Bicêtre neonatal evaluation score [BNES], neonatal multiple organ dysfunction score [NeoMODS]) and mortality and determination of correlation between aortic steal and cerebral damage on initial and follow-up cerebral magnetic resonance imaging (cMRI) were evaluated. Results: Twelve neonates were included, of which 3 died. Per 10 percentage point increase of aortic steal, BNES decreased by 1.64 (95% confidence interval [CI]: 1.28–2.0) points and the maximum observed NeoMODS increased by 1.25 (CI: 0.94–1.57) points. The odds for mortality increased by 2.3 (CI: 1.14–13.67) per 10 percentage point increase of aortic steal. There was a correlation between aortic steal and cerebral damage at baseline (white matter ρ [rho] = 0.34, gray matter ρ = 0.81) and follow-up (white matter ρ = 0.80, gray matter ρ = 0.72). Conclusion: The degree of aortic steal in neonates with VGAM was highly associated with the severity of organ dysfunction, disease progression on cMRI, and mortality.

Vein of Galen malformation is a rare, congenital intracerebral arteriovenous malformation with high morbidity and mortality in neonatal manifestation despite modern interdisciplinary strategies. Currently, there is no valid, noninvasive parameter for early prediction of clinical progression at the bedside. A typical finding of hemodynamically relevant vein of Galen malformation is flow reversal in the descending aorta (aortic steal). Therefore, we developed a new ultrasound method to quantify the percentage of reflux in the preductal aortic isthmus and integrated the measurement method into routine clinical practice. In this study, we retrospectively investigated the association between percentage of aortic steal, organ dysfunction parameters, mortality, and brain injury on magnetic resonance imaging (MRI) at baseline and follow-up in 12 neonates with vein of Galen malformation admitted between January 2021 and June 2022. Statistical analyses showed that the severity of organ dysfunction, mortality, and brain damage on MRI increased with increasing percentage of aortic steal. These correlations and associations showed a linear relationship between percentage reflux in the aortic isthmus and all outcome parameters. The potential of sonographically determined aortic steal to predict clinical progression and short- and long-term outcomes needs to be investigated in larger, prospective cohorts. If this marker proves as promising as the data from this study suggest, it could become an important diagnostic component for management and decision-making in critically ill neonates with vein of Galen malformation.

Vein of Galen (aneurysmal) malformation (VGAM) is an extremely rare disease with an incidence of about 1:58,100 live births per year in Germany [1]. In this cerebrovascular malformation, multiple arteriovenous (AV) fistulas and/or malformations between the persistent median prosencephalic vein of Markowski and various arterial feeding vessels results in intracerebral shunts between the arterial and venous systems [2, 3]. By redistributing cardiac output, these AV shunts reduce perfusion of the brain parenchyma and other vital organs, ultimately producing high-output cardiac failure. At the same time, the high venous cardiac return from the shunt results in volume overload of the right heart and right heart failure [3]. Without intervention, this constellation progresses to refractory multiorgan failure [4, 5]. In spite of modern therapeutic interdisciplinary treatment strategies that have reduced mortality, morbidity remains high in both the short and long term [6‒9].

Although some neonates show signs of right ventricular failure prenatally, postnatal multiorgan failure develops over a period of several hours, days, or even weeks. During intrauterine life, the placental low-pressure system partially compensates the low intracranial arterial resistance [4, 5, 10‒12]. Following postnatal cardiovascular adaptation, cardiac output and blood flow to the lungs, heart, kidney, and the gastrointestinal tract increases under physiological conditions [13]. In VGAM, however, blood flow through the AV malformation increases due to the relatively lower vascular resistance, leading to progressive systemic hypoperfusion with subsequent organ dysfunction [5, 11, 12, 14]. Closure of the ductus arteriosus, which relieves right heart pressure and improves systemic perfusion while open, may lead to acute clinical decompensation [5].

A reliable score to assess disease severity, long-term outcomes, and mortality in severely affected infants is the Bicêtre neonatal evaluation score (BNES) [15]. However, it is unsuitable for early postnatal assessment, as it relies on manifest organ dysfunction, which develops over time [5, 8, 14]. No other early, bedside marker is available to predict the severity of organ dysfunction, mortality, and short-term cerebral damage on magnetic resonance imaging (MRI) [16].

As a manifestation of hemodynamically relevant VGAM, flow reversal in the descending aorta (aortic steal) may occur and is associated with mortality. However, the degree of aortic steal was not quantified in most studies, or the measurement method was evaluated only in terms of mortality in case series using arbitrary cut-off values [4, 8, 14, 17]. The aim of this study was to investigate the association of the percentage of reflux in the preductal aortic isthmus with short-term morbidity, mortality, and cerebral damage on MRI at baseline and follow-up.

Study Setting and Data Collection

All infants with VGAM aged <28 days admitted to the tertiary neonatal intensive care unit of the Sana Hospital Duisburg between January 2021 and June 2022 were included in this retrospective single-center cohort study. Clinical data were collected from the digital patient management systems and the image archiving and communication system (PACS, JiveX Diagnostic Advanced 5.3.0.31, Visus Health IT, Bochum, Germany). The study was approved by the Ethics Committee of the Medical Faculty of the University Hospital Essen (22-10801-BO).

Cardiac Ultrasound and Calculation of Aortic Steal

In January 2021, we introduced a sonographic method to quantify the percentage of reflux in the aortic isthmus into routine clinical practice. Since then, all admitted neonates underwent cardiac ultrasound by a board-certified neonatologist with more than 10 years of experience in neonatal point-of-care US and cardiac US (S.S., F.B.N.) before embolization of the AV malformation. All scans were obtained using one of two high-end ultrasound machines (GE Logiq S8/GE Logiq E10 s R3 (GE Healthcare, Boston, MA, USA) equipped with a sector transducer (6S, GE Healthcare, Boston, MA, USA). First, the aortic arch was visualized in a suprasternal longitudinal plain (Fig. 1). Then, intravascular flow was measured by pulsed Doppler US at the preductal aortic isthmus. For calculation of reflux percentage, the time-averaged maximum velocity above and below the zero flow baseline was automatically measured and averaged during 3–6 pulse curves (S.S.) (online suppl. Fig. S1; for all online suppl. material, see https://doi.org/10.1159/000534132). The percentage of reflux was determined using the volumetric flow formula according to the following calculation:
Aorticsteal=D24×π×TAMAXretrogradeD24×π×TAMAXretrograde+D24×π×TAMAXantegrade=TAMAXretrogradeTAMAXretrograde+TAMAXantegrade,
D = diameter of the vessel.
Fig. 1.

Vein of Galen malformation and aortic steal. a Sagittal T2 weighted MR image of the malformation. b Arterial time-of-flight MR angiography (TOF-MRA) with representation of the arterial feeders (same patients as a). c Systolic retrograde flow (aortic steal) visualized by color Doppler ultrasound in a suprasternal longitudinal plane. 1 = ascending aorta, 2 = truncus brachiocephalicus, 3 = left common carotid artery, 4 = left subclavian artery, 5 = aortic isthmus, 6 = right pulmonary artery. d Aortic steal measured by pulsed Doppler ultrasound at the preductal aortic isthmus. Retrograde flow (aortic steal) above zero flow baseline, antegrade flow below zero flow baseline.

Fig. 1.

Vein of Galen malformation and aortic steal. a Sagittal T2 weighted MR image of the malformation. b Arterial time-of-flight MR angiography (TOF-MRA) with representation of the arterial feeders (same patients as a). c Systolic retrograde flow (aortic steal) visualized by color Doppler ultrasound in a suprasternal longitudinal plane. 1 = ascending aorta, 2 = truncus brachiocephalicus, 3 = left common carotid artery, 4 = left subclavian artery, 5 = aortic isthmus, 6 = right pulmonary artery. d Aortic steal measured by pulsed Doppler ultrasound at the preductal aortic isthmus. Retrograde flow (aortic steal) above zero flow baseline, antegrade flow below zero flow baseline.

Close modal

In addition, septal curvature was assessed in systole in a short-axis view at the level of the papillary muscles and classified as described by King et al. [18]. Type 1 defines a normal round septal curvature, type 2 a flattened septum, and type 3 a leftward convex septum, indicating a suprasystemic right ventricular pressure [18]. In the same plane, left ventricular function was determined in M-mode using the fractional shortening (FS%). The flow direction in the ductus arteriosus and pressure difference across the tricuspid valve were assessed by continuous-wave Doppler in the region with the highest velocity.

Quantification of Organ Dysfunction

Disease severity was quantified using two clinical scores. Maximum clinical impairment before elective intubation for cerebral magnetic resonance imaging (cMRI) and angiography was determined by BNES before elective intubation. The BNES assesses five clinical parameters (cardiac function, respiratory function, cerebral function, renal function, and liver function), forming a total score of 0–21 points that lowers with increasing organ dysfunction (online suppl. Table S1) [15, 19]. The modified neonatal multiple organ dysfunction (NeoMOD) score by Çetinkaya et al. [20] was used to monitor the severity of multiorgan dysfunction over time (online suppl. Table S2). The score ranges between 0 and 16 points and increases with the severity of multiorgan dysfunction. The score was assessed immediately before elective intubation for the first embolization (first cMRT in patients receiving palliative care) and then daily for 7 days. The score on the day after first embolization was defined as NeoMOD1, and the highest score encountered was defined as NeoMODmax.

Magnetic Resonance Imaging

Assessment of the first and follow-up cMRI after 1–10 months was retrospectively performed by an experienced neuroradiologist (N. R. D), blinded to the clinical course using the scoring system for structural brain abnormalities by Woosward et al. [21]. White matter (WM) abnormality was assessed using a three-point scale in five regions and added up to form a total WM score (online suppl. Table S3) [21]. The WM score was graded into normal (5–6 points), mildly abnormal (7–9), moderately abnormal (10–12), and severely abnormal (13–15) [21]. Abnormality of gray matter (GM) was assessed using a three-point scale in three regions. A total GM score was calculated by summing up the points from each region and graded into normal (3–5 points) and abnormal (6–9) [21]. A total score was calculated as the sum of the WM and GM subscores. 19–24 points were considered as severe brain injury.

Poor Outcome

Poor outcome was defined as composite outcome of in-hospital death or severe brain injury on follow-up cMRI (MRI sum score of 19–24 points).

Endovascular Therapy

According to the center-specific standard procedure, a combined therapy with both arterial and venous approaches for precise occlusion of high-flow AV fistulas accurately at the shunting point with coils and/or ethylene vinyl alcohol was performed. Superselective arterial feeder probing in combination with retrograde transvenous approach using the “looping technique” or “kissing microcatheter technique” was used to gain direct access to the inflowing artery at the entry point to the dilated persistent median prosencephalic vein of Markowski [22].

Statistical Analyses

Statistical analysis was performed with SAS Enterprise Guide 8.4 (SAS Institute Inc., Cary, NC, USA) by a blinded observer (N.B.). Categorical variables are summarized as counts and relative frequencies, and continuous variables are presented as median and range. Linear regression analyses on the association of aortic steal with BNES, NeoMOD1, and NeoMODmax were performed. Binary logistic regression was carried out to calculate odds for mortality and the combined poor outcome of death or severe brain injury on cMRI per 10 percentage point increment of aortic steal. Spearman’s correlation coefficient was calculated for aortic steal and cMRI abnormality scores.

Demographic Data and Clinical Features

A total of 12 neonates with a median gestational age of 37 6/7 (range 33 6/7–41 6/7) and a median admission weight of 3,230 g (range 2,220 g–4,250 g) were admitted at a median age of 2 days (range 1–21 days, 5/12 (42%) inborn) (Table 1). Four of 12 (33%) were preterm infants, and 5/12 (42%) weighed less than 2,500 g. According to Lasjaunias, 6/12 (50%) of the AV malformations were choroidal, 4/12 (33%) mural, and 2/12 (17%) mixed-type [15, 19].

Table 1.

Patient characterization and clinical data

Patient No.SexGestational ageWeight, gAge at admission, daysVGAM subtypeAge at 1st embolization/cMRI, daysNo. of interventions during 1st inpatient stayComplications
39 4/7 3,200 21 Mural 23 None 
33 6/7 2,400 Choroidal Palliative care, died 
37 3/7 3,310 Choroidal None 
34 4/7 2,240 Mural Ventricular hemorrhage with posthemorrhagic hydrocephalus, ventriculoperitoneal shunting 
34 4/7 2,340 Mixed Global hemorrhagic infarction of right WM and thalamus with intraventricular hemorrhage 2 days after embolization, palliative care, died 
40 3/7 3,640 Choroidal None 
38 1/7 3,500 Choroidal Palliative care, died 
41 6/7 3,600 14 Mixed 15 None 
39 4/7 3,380 Mural None 
10 34 2/7 2,220 Mural None 
11 37 5/7 2,440 Choroidal Ventricular hemorrhage with posthemorrhagic hydrocephalus, ventriculoperitoneal shunting 
12 40 6/7 3,500 Choroidal None 
Patient No.SexGestational ageWeight, gAge at admission, daysVGAM subtypeAge at 1st embolization/cMRI, daysNo. of interventions during 1st inpatient stayComplications
39 4/7 3,200 21 Mural 23 None 
33 6/7 2,400 Choroidal Palliative care, died 
37 3/7 3,310 Choroidal None 
34 4/7 2,240 Mural Ventricular hemorrhage with posthemorrhagic hydrocephalus, ventriculoperitoneal shunting 
34 4/7 2,340 Mixed Global hemorrhagic infarction of right WM and thalamus with intraventricular hemorrhage 2 days after embolization, palliative care, died 
40 3/7 3,640 Choroidal None 
38 1/7 3,500 Choroidal Palliative care, died 
41 6/7 3,600 14 Mixed 15 None 
39 4/7 3,380 Mural None 
10 34 2/7 2,220 Mural None 
11 37 5/7 2,440 Choroidal Ventricular hemorrhage with posthemorrhagic hydrocephalus, ventriculoperitoneal shunting 
12 40 6/7 3,500 Choroidal None 

F, female; M, male; VGAM, vein of Galen aneurysmal malformation; cMRI, cerebral magnetic resonance imaging.

Two patients received palliative care due to severe brain parenchymal damage at the initial presentation and died within 2 and 5 days after admission, respectively. All other patients underwent endovascular therapy at a median of 5 days (range 1–23 days), 5/10 (50%) within the first 2 days of life. Three/10 infants (30%) experienced serious adverse events during or after embolization: one neonate developed a global hemorrhagic infarction of the right WM and thalamus with intraventricular hemorrhage 2 days after technically successful embolization [23, 24]. This patient died under palliative care on the fourth day of life. Two neonates experienced intraventricular hemorrhage and posthemorrhagic hydrocephalus after the intervention (Table 1). Nine/12 (75%) patients were discharged alive.

Aortic Steal and Cardiac Ultrasound

At least one comprehensive cardiac ultrasound exam was performed on all neonates before embolization. Aortic steal ranged from 5.9 to 89.3% (median 35.7%) (Fig. 2, Table 2). Four patients were examined more than once because of a prolonged interval between hospital admission and intervention with an average deviation of the aortic steal of 3 percentage points (online suppl. Table S4).

Fig. 2.

Different degrees of aortic steal measured by pulsed Doppler ultrasound. Increase from a minimal, barely increased retrograde flow component (a) to a reflux during diastole (b), to a reflux extending into systole (c), to almost complete retrograde flow (d).

Fig. 2.

Different degrees of aortic steal measured by pulsed Doppler ultrasound. Increase from a minimal, barely increased retrograde flow component (a) to a reflux during diastole (b), to a reflux extending into systole (c), to almost complete retrograde flow (d).

Close modal
Table 2.

Cardiac ultrasound, clinical scores, and MRI

Patient No.Aortic steal, %BNESNeoMOD1NeoMODmaxWM grading initialWM grading follow-upGM grading initialGM grading follow-upMortality
8.3 21 Mild None Normal Normal Alive 
71.5 11 11 Severe Abnormal Died 
39 17 Moderate Moderate Abnormal Normal Alive 
89.3 12 12 Mild Severe Abnormal Abnormal Alive 
80.3 10 12 Mild Abnormal Died 
26.9 18 Mild Mild Normal Normal Alive 
76.8 Severe Abnormal Died 
25.3 19 Mild Mild Normal Normal Alive 
5.9 21 None Mild Normal Normal Alive 
10 21 Moderate Mild Normal Normal Alive 
11 59.6 10 10 Mild Severe Normal Abnormal Alive 
12 32.5 18 Mild Mild Normal Normal Alive 
Patient No.Aortic steal, %BNESNeoMOD1NeoMODmaxWM grading initialWM grading follow-upGM grading initialGM grading follow-upMortality
8.3 21 Mild None Normal Normal Alive 
71.5 11 11 Severe Abnormal Died 
39 17 Moderate Moderate Abnormal Normal Alive 
89.3 12 12 Mild Severe Abnormal Abnormal Alive 
80.3 10 12 Mild Abnormal Died 
26.9 18 Mild Mild Normal Normal Alive 
76.8 Severe Abnormal Died 
25.3 19 Mild Mild Normal Normal Alive 
5.9 21 None Mild Normal Normal Alive 
10 21 Moderate Mild Normal Normal Alive 
11 59.6 10 10 Mild Severe Normal Abnormal Alive 
12 32.5 18 Mild Mild Normal Normal Alive 

Septal curvature: 1 = normal round septal curvature, 2 = flattened septum, 3 = leftward convex septum.

BNES, Bicetrê neonatal evaluation score; WM, white matter; GM, gray matter; cMRI, cerebral magnetic resonance imaging.

Ten neonates (83%) showed a flattened or leftward convex intraventricular septum, indicating increased right ventricular pressure. Right-to-left shunt via a patent ductus arteriosus was found in 5/12 (42%) neonates, indicating suprasystemic pulmonary arterial pressure. In patients with measurable tricuspid regurgitation, the pressure difference across the tricuspid valve ranged from 26.8 to 72.4 mm Hg. Almost all patients showed a good left ventricular function (median 33.9%, range 26.8–51%) (online suppl. Table S5).

Organ Dysfunction

Before cMRI and angiography, median BNES score was 17.5 (range, 6–21) and median NeoMOD score was 2.5 (range, 0–9). Linear regression analysis showed a decrease of BNES by 1.64 (confidence interval [CI]: 1.28–2.0) points per 10 percentage point increase of aortic steal (Fig. 3). One day after embolization, the median NeoMOD score (NeoMOD1) increased to 4.5 (range 1–12). The median NeoMODmax was 5 (range 1–12). Linear regression yielded a 1.13 (0.84–1.43) point increase of NeoMOD1 and a 1.25 (CI: 0.94–1.57) point increase of NeoMODmax per 10 percentage point increase of aortic steal (Fig. 3).

Fig. 3.

Association between aortic steal and morbidity. a Association of aortic steal with Bicêtre neonatal evaluation score (BNES). With each 10% increase in aortic steal, BNES decreased by 1.64 (CI: 1.28–2.0) points. b Association of aortic steal with NeoMOD1. With each 10% increase in aortic steal, NeoMOD1 increased by 1.13 (0.84–1.43) points. c Association of aortic steal with NeoMODmax. With each 10% increase in aortic steal, NeoMODmax increased by 1.25 (CI: 0.94–1.57) points. Green asterisk = survivors without severe brain injury (good outcome), red asterisk = survivors with severe brain injury (poor outcome), black asterisk = deceased neonates (poor outcome).

Fig. 3.

Association between aortic steal and morbidity. a Association of aortic steal with Bicêtre neonatal evaluation score (BNES). With each 10% increase in aortic steal, BNES decreased by 1.64 (CI: 1.28–2.0) points. b Association of aortic steal with NeoMOD1. With each 10% increase in aortic steal, NeoMOD1 increased by 1.13 (0.84–1.43) points. c Association of aortic steal with NeoMODmax. With each 10% increase in aortic steal, NeoMODmax increased by 1.25 (CI: 0.94–1.57) points. Green asterisk = survivors without severe brain injury (good outcome), red asterisk = survivors with severe brain injury (poor outcome), black asterisk = deceased neonates (poor outcome).

Close modal

Cerebral Damage on MRI

WM and GM damage on cMRI ranged from no injury/normal to severe injury at both time points (Table 2). Regression analysis showed an increase of cerebral damage (WM, GM, and MRI sum score) with increasing aortic steal at baseline and follow-up (Fig. 4). At initial cMRI, the association and correlation between aortic steal and GM damage were more pronounced than for WM damage (Fig. 4). In contrast, follow-up cMRI showed a stronger correlation between aortic steal and WM damage with a steeper regression slope.

Fig. 4.

Association between aortic steal and cerebral damage on MRI. Association of aortic steal and white matter (WM) score (a), gray matter (GM) score (b), and MRI sum score (c) on the first cMRI. Association of aortic steal and WM score (d), GM score (e), and MRI sum score (f) on follow-up cMRI. *Spearman correlation coefficient; **linear regression slope with 95% CI per 10% aortic steal; WM, white matter; GM, gray matter; cMRI, cerebral magnetic resonance imaging. Green asterisk = survivors without severe cerebral damage (good outcome), red asterisk = survivors with severe cerebral damage (poor outcome), black asterisk = deceased neonates (poor outcome).

Fig. 4.

Association between aortic steal and cerebral damage on MRI. Association of aortic steal and white matter (WM) score (a), gray matter (GM) score (b), and MRI sum score (c) on the first cMRI. Association of aortic steal and WM score (d), GM score (e), and MRI sum score (f) on follow-up cMRI. *Spearman correlation coefficient; **linear regression slope with 95% CI per 10% aortic steal; WM, white matter; GM, gray matter; cMRI, cerebral magnetic resonance imaging. Green asterisk = survivors without severe cerebral damage (good outcome), red asterisk = survivors with severe cerebral damage (poor outcome), black asterisk = deceased neonates (poor outcome).

Close modal

Mortality and Poor Outcome

A total of 9/12 (75%) patients were discharged alive, 3/12 (25%) died, with a strong association between the degree of aortic steal and mortality (Fig. 4). The odds ratio of mortality increased by 2.3 (CI: 1.14–13.67) per 10 percentage point increase in aortic steal. Of survivors, 2/9 (22%) had severe brain injury on follow-up cMRI. All neonates with an aortic steal >59% had a poor outcome and all neonates with an aortic steal <39% had a good outcome. Binary logistic regression analysis to calculate the odds for poor outcome with aortic steal was technically impossible because of a complete separation of data points between good and poor outcome (online suppl. Fig. S2).

This retrospective observational study used a new US-based quantification method of aortic isthmic flow reversal in neonates with VGAM based on the volume flow formula to assess the degree of aortic steal as a noninvasive bedside method for potential prediction of the clinical course and short-term outcomes. We found strong linear correlations of aortic steal with acute organ dysfunction, development of structural brain damage, and mortality.

To date, there is no reliable tool for early prediction of clinical progress and prognosis in VGAM. Current best practice is to base therapy on the severity of heart failure without assessing further objective parameters [5, 16]. However, reliable early prediction of clinical progress would be of particular therapeutic importance, as interventional treatment in a decompensated state is associated with higher complication rates and higher mortality [4]. Further, in some patients, even after stabilization of heart failure, cerebral damage can further progress due to decreased parenchymal perfusion and venous congestion [3].

This study found a close association between aortic steal and morbidity, mortality, and cerebral damage on cMRI at baseline and follow-up, with all outcome parameters worsening with increasing steal. In contrast to previous studies [4, 8, 14, 17], the impact of aortic steal on outcomes was quantified by linear and logistic regression analyses. The complete separation of data points with good outcomes below 39% and poor outcomes above 59% aortic steal suggests that somewhere between or close to these values, a potential optimal cut-point for outcome prediction might be identified in a larger study. From these preliminary data, we draw the conclusion that in neonates with early embolization, an aortic steal below 40% can be considered as a good prognostic factor and an aortic steal above 60% should sensitize care givers and parents for a probable poor outcome with progressive development of structural brain injury even after successful embolization. Another possible scenario is a linear association between brain damage and aortic steal, leading to intermediate outcomes in patients with aortic steal between 39 and 59%, which should be investigated in further studies including long-term neurodevelopmental follow-up.

The observed strong association of aortic steal with outcomes could be explained by a direct association between the degree of blood redistribution in favor of the VGAM and subsequently increasing aortic steal. However, it must be assumed that both the sonographically measurable aortic steal and the effects of blood redistribution on the developing brain are modified by further external and internal factors. For example, patient agitation and examiner experience can affect the technical measurement, whereas drug therapies and changes in volume status exert hemodynamic effects. A relevant left-to-right shunt via an open ductus arteriosus masks isthmic reverse flow through an opposing steal [5, 25]. A strong increase of cardiac output may offset the amount of steal and prevent cerebral damage in some cases. In contrast, cerebral damage may be exacerbated by venous congestion caused by elevated cerebral or central venous pressure, right ventricular failure, and by major interventional complications. In this study, it was impossible to distinguish whether the progressing cerebral injury on follow-up cMRI in survivors was caused by the AV malformation itself or secondary damage from interventional complications [23, 24]. Major interventional complications occurred only in the high-risk group, making it difficult to disentangle the interaction between aortic steal, interventional risks, and structural brain damage.

Our study is limited by the small sample size and retrospective, single-center study design. Due to the retrospective study design, it was not possible to establish uniform measurement time points. However, a separate comparison of multiple measurements suggests that the intraobserver variability of aortic steal is negligible in pre-interventional serial exams. Additional prospective studies should examine the relevance of different measurement time points and interobserver reliability. Furthermore, the diagnosis of brain damage via cMRI suffers from the unknown relationship between detectable structural damage and functional long-term neurological outcomes. Additional gradations of functional neurodevelopmental outcomes can be hypothesized that were not captured with this study design.

The potential of aortic steal to predict the clinical course and short- and long-term outcomes must be evaluated in larger, multicentric cohorts. If this marker turns out to be as promising as the data from this study suggest, it may become an important diagnostic building block to inform decision-making in critically ill neonates with VGAM. In this context, it seems also worthwhile to investigate if prenatal measurement of aortic steal can contribute to decision-making.

Sonographically determined aortic steal is associated with organ dysfunction and mortality at initial hospitalization and with cerebral damage on cMRI at baseline and follow-up. Noninvasive bedside measurement of aortic steal may allow physicians to identify high-risk neonates, monitor them closely, and direct therapeutic measures accordingly.

The study was approved by the Medical Ethics Committee of the Medical Faculty of the University Hospital Essen (22-10801-BO). As this study did not fall under the Medical Research Involving Human Subjects Act, clinically obtained anonymized data were used, and the Medical Ethic Committee waived the need for informed consent.

Friedhelm Brassel declares the following competing interest: shareholder of the company Bentley InnoMed GmbH, Hechingen, Germany. All other authors have no conflicts of interest to declare.

No funding was received for this study.

Simone Schwarz contributed to design methodology, investigation, data curation, formal analysis, validation, drafting, and writing/editing of the initial original manuscript of this study. Francisco Brevis Nuñez contributed to investigation, formal analysis, supervision, and reviewing of the manuscript of this study. Nikola R Dürr and Martin Schlunz-Hendann contributed to investigation, data curation, formal analysis, methodology, validation, visualization, and reviewing of the manuscript of this study. Friedhelm Brassel contributed to data curation, formal analysis, methodology, project administration, supervision, validation, and reviewing of the manuscript of this study. Ursula Felderhoff-Müser contributed to formal analysis, resources, supervision, validation, and reviewing the manuscript of this study. Christian Dohna-Schwake contributed to conceptualization, formal analysis, resources, supervision, and reviewing the manuscript of this study. Nora Bruns contributed to methodology, investigation, conceptualization, data curation, formal analysis, project administration, supervision, validation, visualization, and reviewing the manuscript of this study.

The data that support the findings of this study are not publicly available due to privacy concerns. Requests for data availability can be made via the corresponding author.

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